Exertional breathlessness in pulmonary arterial hypertensio

Recruitment & Eligibility

Status: ot yet recruiting

Sex: All

Target Recruitment: 15

Inclusion Criteria

• Patients with Group 1 PAH as per 6th World Symposium on Pulmonary Hypertension
• Exertional dyspnoea (WHO functional class II and III)
• Men and women
• Aged 18 years or over

Exclusion Criteria

• Group 2, 3, 4, 5 pulmonary hypertension
• Six minute walk distance < 300m
• Dyspnoea at rest (WHO functional class IV)
• Significant left ventricular dysfunction (Left ventricular ejection fraction <40% or significant diastolic dysfunction)
• Obstructive or restrictive lung disease
o Pulmonary fibrosis or emphysema on chest radiograph or computed tomography scan
o Total lung capacity <80% of predicted
o Forced expiratory volume in first second of expiration/ forced vital capacity <70%
o Currently taking regular inhaled therapy for airways disease
• Serious co-morbidities that may contribute to dyspnoea and/or reduce exercise capacity including:
o Severe cardiac disease (e.g., heart failure, coronary artery disease)
o Severe respiratory disease other than PAH (e.g., chronic obstructive pulmonary disease, interstitial lung disease)
o Severe obesity (Body mass index > 35 kg/m2)
o Severe orthopaedic impairment or rheumatologic/ connective tissue disease
o Significant neurological disease
o Significant renal or liver disease
o Infection or pyrexial illness
o Uncontrolled thyroid disorders
o Current active treatment for cancer
• Presence of any contraindications to cardiopulmonary exercise testing
o Unstable angina or recent acute myocardial infarction
o Uncontrolled arrhythmias causing symptoms or haemodynamic compromise
o Symptomatic severe aortic stenosis
o Oxygen saturation <85% at rest on room air
o Uncontrolled heart failure
o Uncontrolled asthma
o Uncontrolled thyroid disorders
o Mental impairment leading to inability to cooperate
o Long-term oxygen therapy
• Current pregnancy
• Allergy or intolerance to medications used in the study (iloprost)
• Contra-indication to study medication (iloprost)
• Current users of recreational drugs
• Current abusers of alcohol
• Inability to fully or appropriately provide consent (e.g., language issue, reading capability)
• Underlying medical conditions, which in the opinion of the Investigator place the participant at unacceptably high risk for participating in the study.